Haemophilia

Question 1

What is the deficiency in haemophilia A

Answer 1

Factor VIII deficiency

Question 2

What is the deficiency in haemophilia B

Answer 2

Factor IX deficiency

Question 3

Clinical features of haemophilia

Answer 3

Mild moderate or severe depending on level of factor. Bleeding into muscle or joints

Question 4

Age of presentation

Answer 4

Most around 1st year of life when crawling or walking
40% present in neonatal period with intracranial haemorrhage or prolonged bleeding

Question 5

Management of haemophilia

Answer 5

Recombinant factor given IV when any bleeding or preemptively for surgery. Prophylactically in severe haemophilia A

Question 6

What should be avoided

Answer 6

IM, NSAID and aspirin

Question 7

Complications of haemophilia

Answer 7

Antibodies to factor VIII or IX which reduce or completely inhibit effect of treatment.
Transfusion transmitted infection
Difficult vascular access

Question 8

What is haemophilia

Answer 8

Where the blood does not clot properly due to clotting factor deficiency